Slide 1

Case 26: 25 y.o. Male. Right shoulder.
Keloid? Hypertrophic scar? Lichen simplex
chronicus?

On examination
On Examination
• Approximately 5x4cm
dermal nodule
• Firm texture
• right shoulder
• no lymphadenopathy.
The initial impression was this was a cyst and excision was organised by his
GP on a General Surgery Minor ops list.

Histopathology
• Unencapsulated spindle cell lesion
• composed of short fascicular arrays in a
storiform pattern
• nestled within the mid-dermis extending
widely into sub-cutis.
• Scattered melanophages were noted.
Immunolabelling showed strong positivity
for CD34 and very focal nonspecific
positivity for SMA & Factor XIIIa

CD34

S100

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma
Features consistent with a pigmented dermatofibrosarcoma
protuberans or Bednar tumour.

Bednar Tumour
Pigmented DFSP varies from ordinary DFSP by the presence of
non neoplastic melanin- laden dendritic cells; it is otherwise
morphologically and clinically identical to ordinary DFSP
Czech pathologist, Blahoslav
Bednar
(19161998),
first
described the Pigmented DFSP in
1957 while describing a group of
cutaneous tumours demonstrating
prominent storiform pattern and
melanin pigment which were
thought to be a variant of
Neurofibroma.

Less than 5% of dermatofibrosarcoma protuberans (DFSP)
tumours are pigmented.

Dermatofibrosarcoma Protuberans
• Rare- 1 case per million per year
• Cutaneous soft tissue sarcoma
originating from Fibroblasts
• 90% are low grade, 10% contain an
additional high grade sarcomatous
component therefore it is considered to
be of intermediate malignancy
• <4% of tumours can
metastasise/transform
fibrosarcomatous

Pathophysiology
• 90% of DFSP have the translocation between
Ch17 and Ch22 [t(17:22)]
• Creates a fusion between a collagen gene
and platelet derived growth

• Resultant fusion gene creates platelet
derived growth factor (PDGF)
• Fibroblasts carry the platelet-derived growth
factor receptor β
• Malignant cells produce PDGF which
stimulates fibroblast growth

• As malignant cells develop, tumour enlarges

Mortality/morbidity
• locally aggressive with high
recurrence rate
• 5-year survival - 99.2%
• metastasis rare (only 1-4%
reported)

Prognosis & Adjunct Therapy
• Tumour depth is the only factor
associated with disease- free survival
• Follow-up should involve
• Metastatic/ unresectable tumours can
be treated with Imatinib mesylate
(Glivec) if t(17:22) positive
• There is a role for radiotherapy after
surgery

Management
BB was referred to
the Skin Cancer MDT
for
further
management
On examination, he
had his original scar
on the right shoulder
as well as remnants
of his DFSP

Medical Treatment
• Radiation therapy
– margins positive
– Postoperative – reduce risk of recurrence

Excision
Wide Local Excision Vs.

Mohs Surgery

Goal is achieve clear surgical margins with some form of complete circumferential
and peripheral deep margin assessment before reconstruction

Management of DFSP

Matin, R.N., Acland, K.M. and Williams, H.C. (2012) British Journal of Dermatology,
167: 6–9.

No randomized controlled trials exist, probably due to the
rarity and varied presentation of DFSP.
A recent published review of the literature highlighted that
evidence for superiority of either procedure was limited.

Medical Treatment
• Imatinib
– inhibitor protein-tyrosine kinases
– platelet-derived growth factor (PDGF)
receptors
– unresectable, recurrent, metastatic DFSP
– preoperative therapy
• decrease tumour load
• promote tumour cell apoptosis
• reduce the extent of surgery.


Slide 2

Case 26: 25 y.o. Male. Right shoulder.
Keloid? Hypertrophic scar? Lichen simplex
chronicus?

On examination
On Examination
• Approximately 5x4cm
dermal nodule
• Firm texture
• right shoulder
• no lymphadenopathy.
The initial impression was this was a cyst and excision was organised by his
GP on a General Surgery Minor ops list.

Histopathology
• Unencapsulated spindle cell lesion
• composed of short fascicular arrays in a
storiform pattern
• nestled within the mid-dermis extending
widely into sub-cutis.
• Scattered melanophages were noted.
Immunolabelling showed strong positivity
for CD34 and very focal nonspecific
positivity for SMA & Factor XIIIa

CD34

S100

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma
Features consistent with a pigmented dermatofibrosarcoma
protuberans or Bednar tumour.

Bednar Tumour
Pigmented DFSP varies from ordinary DFSP by the presence of
non neoplastic melanin- laden dendritic cells; it is otherwise
morphologically and clinically identical to ordinary DFSP
Czech pathologist, Blahoslav
Bednar
(19161998),
first
described the Pigmented DFSP in
1957 while describing a group of
cutaneous tumours demonstrating
prominent storiform pattern and
melanin pigment which were
thought to be a variant of
Neurofibroma.

Less than 5% of dermatofibrosarcoma protuberans (DFSP)
tumours are pigmented.

Dermatofibrosarcoma Protuberans
• Rare- 1 case per million per year
• Cutaneous soft tissue sarcoma
originating from Fibroblasts
• 90% are low grade, 10% contain an
additional high grade sarcomatous
component therefore it is considered to
be of intermediate malignancy
• <4% of tumours can
metastasise/transform
fibrosarcomatous

Pathophysiology
• 90% of DFSP have the translocation between
Ch17 and Ch22 [t(17:22)]
• Creates a fusion between a collagen gene
and platelet derived growth

• Resultant fusion gene creates platelet
derived growth factor (PDGF)
• Fibroblasts carry the platelet-derived growth
factor receptor β
• Malignant cells produce PDGF which
stimulates fibroblast growth

• As malignant cells develop, tumour enlarges

Mortality/morbidity
• locally aggressive with high
recurrence rate
• 5-year survival - 99.2%
• metastasis rare (only 1-4%
reported)

Prognosis & Adjunct Therapy
• Tumour depth is the only factor
associated with disease- free survival
• Follow-up should involve
• Metastatic/ unresectable tumours can
be treated with Imatinib mesylate
(Glivec) if t(17:22) positive
• There is a role for radiotherapy after
surgery

Management
BB was referred to
the Skin Cancer MDT
for
further
management
On examination, he
had his original scar
on the right shoulder
as well as remnants
of his DFSP

Medical Treatment
• Radiation therapy
– margins positive
– Postoperative – reduce risk of recurrence

Excision
Wide Local Excision Vs.

Mohs Surgery

Goal is achieve clear surgical margins with some form of complete circumferential
and peripheral deep margin assessment before reconstruction

Management of DFSP

Matin, R.N., Acland, K.M. and Williams, H.C. (2012) British Journal of Dermatology,
167: 6–9.

No randomized controlled trials exist, probably due to the
rarity and varied presentation of DFSP.
A recent published review of the literature highlighted that
evidence for superiority of either procedure was limited.

Medical Treatment
• Imatinib
– inhibitor protein-tyrosine kinases
– platelet-derived growth factor (PDGF)
receptors
– unresectable, recurrent, metastatic DFSP
– preoperative therapy
• decrease tumour load
• promote tumour cell apoptosis
• reduce the extent of surgery.


Slide 3

Case 26: 25 y.o. Male. Right shoulder.
Keloid? Hypertrophic scar? Lichen simplex
chronicus?

On examination
On Examination
• Approximately 5x4cm
dermal nodule
• Firm texture
• right shoulder
• no lymphadenopathy.
The initial impression was this was a cyst and excision was organised by his
GP on a General Surgery Minor ops list.

Histopathology
• Unencapsulated spindle cell lesion
• composed of short fascicular arrays in a
storiform pattern
• nestled within the mid-dermis extending
widely into sub-cutis.
• Scattered melanophages were noted.
Immunolabelling showed strong positivity
for CD34 and very focal nonspecific
positivity for SMA & Factor XIIIa

CD34

S100

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma
Features consistent with a pigmented dermatofibrosarcoma
protuberans or Bednar tumour.

Bednar Tumour
Pigmented DFSP varies from ordinary DFSP by the presence of
non neoplastic melanin- laden dendritic cells; it is otherwise
morphologically and clinically identical to ordinary DFSP
Czech pathologist, Blahoslav
Bednar
(19161998),
first
described the Pigmented DFSP in
1957 while describing a group of
cutaneous tumours demonstrating
prominent storiform pattern and
melanin pigment which were
thought to be a variant of
Neurofibroma.

Less than 5% of dermatofibrosarcoma protuberans (DFSP)
tumours are pigmented.

Dermatofibrosarcoma Protuberans
• Rare- 1 case per million per year
• Cutaneous soft tissue sarcoma
originating from Fibroblasts
• 90% are low grade, 10% contain an
additional high grade sarcomatous
component therefore it is considered to
be of intermediate malignancy
• <4% of tumours can
metastasise/transform
fibrosarcomatous

Pathophysiology
• 90% of DFSP have the translocation between
Ch17 and Ch22 [t(17:22)]
• Creates a fusion between a collagen gene
and platelet derived growth

• Resultant fusion gene creates platelet
derived growth factor (PDGF)
• Fibroblasts carry the platelet-derived growth
factor receptor β
• Malignant cells produce PDGF which
stimulates fibroblast growth

• As malignant cells develop, tumour enlarges

Mortality/morbidity
• locally aggressive with high
recurrence rate
• 5-year survival - 99.2%
• metastasis rare (only 1-4%
reported)

Prognosis & Adjunct Therapy
• Tumour depth is the only factor
associated with disease- free survival
• Follow-up should involve
• Metastatic/ unresectable tumours can
be treated with Imatinib mesylate
(Glivec) if t(17:22) positive
• There is a role for radiotherapy after
surgery

Management
BB was referred to
the Skin Cancer MDT
for
further
management
On examination, he
had his original scar
on the right shoulder
as well as remnants
of his DFSP

Medical Treatment
• Radiation therapy
– margins positive
– Postoperative – reduce risk of recurrence

Excision
Wide Local Excision Vs.

Mohs Surgery

Goal is achieve clear surgical margins with some form of complete circumferential
and peripheral deep margin assessment before reconstruction

Management of DFSP

Matin, R.N., Acland, K.M. and Williams, H.C. (2012) British Journal of Dermatology,
167: 6–9.

No randomized controlled trials exist, probably due to the
rarity and varied presentation of DFSP.
A recent published review of the literature highlighted that
evidence for superiority of either procedure was limited.

Medical Treatment
• Imatinib
– inhibitor protein-tyrosine kinases
– platelet-derived growth factor (PDGF)
receptors
– unresectable, recurrent, metastatic DFSP
– preoperative therapy
• decrease tumour load
• promote tumour cell apoptosis
• reduce the extent of surgery.


Slide 4

Case 26: 25 y.o. Male. Right shoulder.
Keloid? Hypertrophic scar? Lichen simplex
chronicus?

On examination
On Examination
• Approximately 5x4cm
dermal nodule
• Firm texture
• right shoulder
• no lymphadenopathy.
The initial impression was this was a cyst and excision was organised by his
GP on a General Surgery Minor ops list.

Histopathology
• Unencapsulated spindle cell lesion
• composed of short fascicular arrays in a
storiform pattern
• nestled within the mid-dermis extending
widely into sub-cutis.
• Scattered melanophages were noted.
Immunolabelling showed strong positivity
for CD34 and very focal nonspecific
positivity for SMA & Factor XIIIa

CD34

S100

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma
Features consistent with a pigmented dermatofibrosarcoma
protuberans or Bednar tumour.

Bednar Tumour
Pigmented DFSP varies from ordinary DFSP by the presence of
non neoplastic melanin- laden dendritic cells; it is otherwise
morphologically and clinically identical to ordinary DFSP
Czech pathologist, Blahoslav
Bednar
(19161998),
first
described the Pigmented DFSP in
1957 while describing a group of
cutaneous tumours demonstrating
prominent storiform pattern and
melanin pigment which were
thought to be a variant of
Neurofibroma.

Less than 5% of dermatofibrosarcoma protuberans (DFSP)
tumours are pigmented.

Dermatofibrosarcoma Protuberans
• Rare- 1 case per million per year
• Cutaneous soft tissue sarcoma
originating from Fibroblasts
• 90% are low grade, 10% contain an
additional high grade sarcomatous
component therefore it is considered to
be of intermediate malignancy
• <4% of tumours can
metastasise/transform
fibrosarcomatous

Pathophysiology
• 90% of DFSP have the translocation between
Ch17 and Ch22 [t(17:22)]
• Creates a fusion between a collagen gene
and platelet derived growth

• Resultant fusion gene creates platelet
derived growth factor (PDGF)
• Fibroblasts carry the platelet-derived growth
factor receptor β
• Malignant cells produce PDGF which
stimulates fibroblast growth

• As malignant cells develop, tumour enlarges

Mortality/morbidity
• locally aggressive with high
recurrence rate
• 5-year survival - 99.2%
• metastasis rare (only 1-4%
reported)

Prognosis & Adjunct Therapy
• Tumour depth is the only factor
associated with disease- free survival
• Follow-up should involve
• Metastatic/ unresectable tumours can
be treated with Imatinib mesylate
(Glivec) if t(17:22) positive
• There is a role for radiotherapy after
surgery

Management
BB was referred to
the Skin Cancer MDT
for
further
management
On examination, he
had his original scar
on the right shoulder
as well as remnants
of his DFSP

Medical Treatment
• Radiation therapy
– margins positive
– Postoperative – reduce risk of recurrence

Excision
Wide Local Excision Vs.

Mohs Surgery

Goal is achieve clear surgical margins with some form of complete circumferential
and peripheral deep margin assessment before reconstruction

Management of DFSP

Matin, R.N., Acland, K.M. and Williams, H.C. (2012) British Journal of Dermatology,
167: 6–9.

No randomized controlled trials exist, probably due to the
rarity and varied presentation of DFSP.
A recent published review of the literature highlighted that
evidence for superiority of either procedure was limited.

Medical Treatment
• Imatinib
– inhibitor protein-tyrosine kinases
– platelet-derived growth factor (PDGF)
receptors
– unresectable, recurrent, metastatic DFSP
– preoperative therapy
• decrease tumour load
• promote tumour cell apoptosis
• reduce the extent of surgery.


Slide 5

Case 26: 25 y.o. Male. Right shoulder.
Keloid? Hypertrophic scar? Lichen simplex
chronicus?

On examination
On Examination
• Approximately 5x4cm
dermal nodule
• Firm texture
• right shoulder
• no lymphadenopathy.
The initial impression was this was a cyst and excision was organised by his
GP on a General Surgery Minor ops list.

Histopathology
• Unencapsulated spindle cell lesion
• composed of short fascicular arrays in a
storiform pattern
• nestled within the mid-dermis extending
widely into sub-cutis.
• Scattered melanophages were noted.
Immunolabelling showed strong positivity
for CD34 and very focal nonspecific
positivity for SMA & Factor XIIIa

CD34

S100

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma
Features consistent with a pigmented dermatofibrosarcoma
protuberans or Bednar tumour.

Bednar Tumour
Pigmented DFSP varies from ordinary DFSP by the presence of
non neoplastic melanin- laden dendritic cells; it is otherwise
morphologically and clinically identical to ordinary DFSP
Czech pathologist, Blahoslav
Bednar
(19161998),
first
described the Pigmented DFSP in
1957 while describing a group of
cutaneous tumours demonstrating
prominent storiform pattern and
melanin pigment which were
thought to be a variant of
Neurofibroma.

Less than 5% of dermatofibrosarcoma protuberans (DFSP)
tumours are pigmented.

Dermatofibrosarcoma Protuberans
• Rare- 1 case per million per year
• Cutaneous soft tissue sarcoma
originating from Fibroblasts
• 90% are low grade, 10% contain an
additional high grade sarcomatous
component therefore it is considered to
be of intermediate malignancy
• <4% of tumours can
metastasise/transform
fibrosarcomatous

Pathophysiology
• 90% of DFSP have the translocation between
Ch17 and Ch22 [t(17:22)]
• Creates a fusion between a collagen gene
and platelet derived growth

• Resultant fusion gene creates platelet
derived growth factor (PDGF)
• Fibroblasts carry the platelet-derived growth
factor receptor β
• Malignant cells produce PDGF which
stimulates fibroblast growth

• As malignant cells develop, tumour enlarges

Mortality/morbidity
• locally aggressive with high
recurrence rate
• 5-year survival - 99.2%
• metastasis rare (only 1-4%
reported)

Prognosis & Adjunct Therapy
• Tumour depth is the only factor
associated with disease- free survival
• Follow-up should involve
• Metastatic/ unresectable tumours can
be treated with Imatinib mesylate
(Glivec) if t(17:22) positive
• There is a role for radiotherapy after
surgery

Management
BB was referred to
the Skin Cancer MDT
for
further
management
On examination, he
had his original scar
on the right shoulder
as well as remnants
of his DFSP

Medical Treatment
• Radiation therapy
– margins positive
– Postoperative – reduce risk of recurrence

Excision
Wide Local Excision Vs.

Mohs Surgery

Goal is achieve clear surgical margins with some form of complete circumferential
and peripheral deep margin assessment before reconstruction

Management of DFSP

Matin, R.N., Acland, K.M. and Williams, H.C. (2012) British Journal of Dermatology,
167: 6–9.

No randomized controlled trials exist, probably due to the
rarity and varied presentation of DFSP.
A recent published review of the literature highlighted that
evidence for superiority of either procedure was limited.

Medical Treatment
• Imatinib
– inhibitor protein-tyrosine kinases
– platelet-derived growth factor (PDGF)
receptors
– unresectable, recurrent, metastatic DFSP
– preoperative therapy
• decrease tumour load
• promote tumour cell apoptosis
• reduce the extent of surgery.


Slide 6

Case 26: 25 y.o. Male. Right shoulder.
Keloid? Hypertrophic scar? Lichen simplex
chronicus?

On examination
On Examination
• Approximately 5x4cm
dermal nodule
• Firm texture
• right shoulder
• no lymphadenopathy.
The initial impression was this was a cyst and excision was organised by his
GP on a General Surgery Minor ops list.

Histopathology
• Unencapsulated spindle cell lesion
• composed of short fascicular arrays in a
storiform pattern
• nestled within the mid-dermis extending
widely into sub-cutis.
• Scattered melanophages were noted.
Immunolabelling showed strong positivity
for CD34 and very focal nonspecific
positivity for SMA & Factor XIIIa

CD34

S100

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma
Features consistent with a pigmented dermatofibrosarcoma
protuberans or Bednar tumour.

Bednar Tumour
Pigmented DFSP varies from ordinary DFSP by the presence of
non neoplastic melanin- laden dendritic cells; it is otherwise
morphologically and clinically identical to ordinary DFSP
Czech pathologist, Blahoslav
Bednar
(19161998),
first
described the Pigmented DFSP in
1957 while describing a group of
cutaneous tumours demonstrating
prominent storiform pattern and
melanin pigment which were
thought to be a variant of
Neurofibroma.

Less than 5% of dermatofibrosarcoma protuberans (DFSP)
tumours are pigmented.

Dermatofibrosarcoma Protuberans
• Rare- 1 case per million per year
• Cutaneous soft tissue sarcoma
originating from Fibroblasts
• 90% are low grade, 10% contain an
additional high grade sarcomatous
component therefore it is considered to
be of intermediate malignancy
• <4% of tumours can
metastasise/transform
fibrosarcomatous

Pathophysiology
• 90% of DFSP have the translocation between
Ch17 and Ch22 [t(17:22)]
• Creates a fusion between a collagen gene
and platelet derived growth

• Resultant fusion gene creates platelet
derived growth factor (PDGF)
• Fibroblasts carry the platelet-derived growth
factor receptor β
• Malignant cells produce PDGF which
stimulates fibroblast growth

• As malignant cells develop, tumour enlarges

Mortality/morbidity
• locally aggressive with high
recurrence rate
• 5-year survival - 99.2%
• metastasis rare (only 1-4%
reported)

Prognosis & Adjunct Therapy
• Tumour depth is the only factor
associated with disease- free survival
• Follow-up should involve
• Metastatic/ unresectable tumours can
be treated with Imatinib mesylate
(Glivec) if t(17:22) positive
• There is a role for radiotherapy after
surgery

Management
BB was referred to
the Skin Cancer MDT
for
further
management
On examination, he
had his original scar
on the right shoulder
as well as remnants
of his DFSP

Medical Treatment
• Radiation therapy
– margins positive
– Postoperative – reduce risk of recurrence

Excision
Wide Local Excision Vs.

Mohs Surgery

Goal is achieve clear surgical margins with some form of complete circumferential
and peripheral deep margin assessment before reconstruction

Management of DFSP

Matin, R.N., Acland, K.M. and Williams, H.C. (2012) British Journal of Dermatology,
167: 6–9.

No randomized controlled trials exist, probably due to the
rarity and varied presentation of DFSP.
A recent published review of the literature highlighted that
evidence for superiority of either procedure was limited.

Medical Treatment
• Imatinib
– inhibitor protein-tyrosine kinases
– platelet-derived growth factor (PDGF)
receptors
– unresectable, recurrent, metastatic DFSP
– preoperative therapy
• decrease tumour load
• promote tumour cell apoptosis
• reduce the extent of surgery.


Slide 7

Case 26: 25 y.o. Male. Right shoulder.
Keloid? Hypertrophic scar? Lichen simplex
chronicus?

On examination
On Examination
• Approximately 5x4cm
dermal nodule
• Firm texture
• right shoulder
• no lymphadenopathy.
The initial impression was this was a cyst and excision was organised by his
GP on a General Surgery Minor ops list.

Histopathology
• Unencapsulated spindle cell lesion
• composed of short fascicular arrays in a
storiform pattern
• nestled within the mid-dermis extending
widely into sub-cutis.
• Scattered melanophages were noted.
Immunolabelling showed strong positivity
for CD34 and very focal nonspecific
positivity for SMA & Factor XIIIa

CD34

S100

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma
Features consistent with a pigmented dermatofibrosarcoma
protuberans or Bednar tumour.

Bednar Tumour
Pigmented DFSP varies from ordinary DFSP by the presence of
non neoplastic melanin- laden dendritic cells; it is otherwise
morphologically and clinically identical to ordinary DFSP
Czech pathologist, Blahoslav
Bednar
(19161998),
first
described the Pigmented DFSP in
1957 while describing a group of
cutaneous tumours demonstrating
prominent storiform pattern and
melanin pigment which were
thought to be a variant of
Neurofibroma.

Less than 5% of dermatofibrosarcoma protuberans (DFSP)
tumours are pigmented.

Dermatofibrosarcoma Protuberans
• Rare- 1 case per million per year
• Cutaneous soft tissue sarcoma
originating from Fibroblasts
• 90% are low grade, 10% contain an
additional high grade sarcomatous
component therefore it is considered to
be of intermediate malignancy
• <4% of tumours can
metastasise/transform
fibrosarcomatous

Pathophysiology
• 90% of DFSP have the translocation between
Ch17 and Ch22 [t(17:22)]
• Creates a fusion between a collagen gene
and platelet derived growth

• Resultant fusion gene creates platelet
derived growth factor (PDGF)
• Fibroblasts carry the platelet-derived growth
factor receptor β
• Malignant cells produce PDGF which
stimulates fibroblast growth

• As malignant cells develop, tumour enlarges

Mortality/morbidity
• locally aggressive with high
recurrence rate
• 5-year survival - 99.2%
• metastasis rare (only 1-4%
reported)

Prognosis & Adjunct Therapy
• Tumour depth is the only factor
associated with disease- free survival
• Follow-up should involve
• Metastatic/ unresectable tumours can
be treated with Imatinib mesylate
(Glivec) if t(17:22) positive
• There is a role for radiotherapy after
surgery

Management
BB was referred to
the Skin Cancer MDT
for
further
management
On examination, he
had his original scar
on the right shoulder
as well as remnants
of his DFSP

Medical Treatment
• Radiation therapy
– margins positive
– Postoperative – reduce risk of recurrence

Excision
Wide Local Excision Vs.

Mohs Surgery

Goal is achieve clear surgical margins with some form of complete circumferential
and peripheral deep margin assessment before reconstruction

Management of DFSP

Matin, R.N., Acland, K.M. and Williams, H.C. (2012) British Journal of Dermatology,
167: 6–9.

No randomized controlled trials exist, probably due to the
rarity and varied presentation of DFSP.
A recent published review of the literature highlighted that
evidence for superiority of either procedure was limited.

Medical Treatment
• Imatinib
– inhibitor protein-tyrosine kinases
– platelet-derived growth factor (PDGF)
receptors
– unresectable, recurrent, metastatic DFSP
– preoperative therapy
• decrease tumour load
• promote tumour cell apoptosis
• reduce the extent of surgery.


Slide 8

Case 26: 25 y.o. Male. Right shoulder.
Keloid? Hypertrophic scar? Lichen simplex
chronicus?

On examination
On Examination
• Approximately 5x4cm
dermal nodule
• Firm texture
• right shoulder
• no lymphadenopathy.
The initial impression was this was a cyst and excision was organised by his
GP on a General Surgery Minor ops list.

Histopathology
• Unencapsulated spindle cell lesion
• composed of short fascicular arrays in a
storiform pattern
• nestled within the mid-dermis extending
widely into sub-cutis.
• Scattered melanophages were noted.
Immunolabelling showed strong positivity
for CD34 and very focal nonspecific
positivity for SMA & Factor XIIIa

CD34

S100

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma
Features consistent with a pigmented dermatofibrosarcoma
protuberans or Bednar tumour.

Bednar Tumour
Pigmented DFSP varies from ordinary DFSP by the presence of
non neoplastic melanin- laden dendritic cells; it is otherwise
morphologically and clinically identical to ordinary DFSP
Czech pathologist, Blahoslav
Bednar
(19161998),
first
described the Pigmented DFSP in
1957 while describing a group of
cutaneous tumours demonstrating
prominent storiform pattern and
melanin pigment which were
thought to be a variant of
Neurofibroma.

Less than 5% of dermatofibrosarcoma protuberans (DFSP)
tumours are pigmented.

Dermatofibrosarcoma Protuberans
• Rare- 1 case per million per year
• Cutaneous soft tissue sarcoma
originating from Fibroblasts
• 90% are low grade, 10% contain an
additional high grade sarcomatous
component therefore it is considered to
be of intermediate malignancy
• <4% of tumours can
metastasise/transform
fibrosarcomatous

Pathophysiology
• 90% of DFSP have the translocation between
Ch17 and Ch22 [t(17:22)]
• Creates a fusion between a collagen gene
and platelet derived growth

• Resultant fusion gene creates platelet
derived growth factor (PDGF)
• Fibroblasts carry the platelet-derived growth
factor receptor β
• Malignant cells produce PDGF which
stimulates fibroblast growth

• As malignant cells develop, tumour enlarges

Mortality/morbidity
• locally aggressive with high
recurrence rate
• 5-year survival - 99.2%
• metastasis rare (only 1-4%
reported)

Prognosis & Adjunct Therapy
• Tumour depth is the only factor
associated with disease- free survival
• Follow-up should involve
• Metastatic/ unresectable tumours can
be treated with Imatinib mesylate
(Glivec) if t(17:22) positive
• There is a role for radiotherapy after
surgery

Management
BB was referred to
the Skin Cancer MDT
for
further
management
On examination, he
had his original scar
on the right shoulder
as well as remnants
of his DFSP

Medical Treatment
• Radiation therapy
– margins positive
– Postoperative – reduce risk of recurrence

Excision
Wide Local Excision Vs.

Mohs Surgery

Goal is achieve clear surgical margins with some form of complete circumferential
and peripheral deep margin assessment before reconstruction

Management of DFSP

Matin, R.N., Acland, K.M. and Williams, H.C. (2012) British Journal of Dermatology,
167: 6–9.

No randomized controlled trials exist, probably due to the
rarity and varied presentation of DFSP.
A recent published review of the literature highlighted that
evidence for superiority of either procedure was limited.

Medical Treatment
• Imatinib
– inhibitor protein-tyrosine kinases
– platelet-derived growth factor (PDGF)
receptors
– unresectable, recurrent, metastatic DFSP
– preoperative therapy
• decrease tumour load
• promote tumour cell apoptosis
• reduce the extent of surgery.


Slide 9

Case 26: 25 y.o. Male. Right shoulder.
Keloid? Hypertrophic scar? Lichen simplex
chronicus?

On examination
On Examination
• Approximately 5x4cm
dermal nodule
• Firm texture
• right shoulder
• no lymphadenopathy.
The initial impression was this was a cyst and excision was organised by his
GP on a General Surgery Minor ops list.

Histopathology
• Unencapsulated spindle cell lesion
• composed of short fascicular arrays in a
storiform pattern
• nestled within the mid-dermis extending
widely into sub-cutis.
• Scattered melanophages were noted.
Immunolabelling showed strong positivity
for CD34 and very focal nonspecific
positivity for SMA & Factor XIIIa

CD34

S100

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma
Features consistent with a pigmented dermatofibrosarcoma
protuberans or Bednar tumour.

Bednar Tumour
Pigmented DFSP varies from ordinary DFSP by the presence of
non neoplastic melanin- laden dendritic cells; it is otherwise
morphologically and clinically identical to ordinary DFSP
Czech pathologist, Blahoslav
Bednar
(19161998),
first
described the Pigmented DFSP in
1957 while describing a group of
cutaneous tumours demonstrating
prominent storiform pattern and
melanin pigment which were
thought to be a variant of
Neurofibroma.

Less than 5% of dermatofibrosarcoma protuberans (DFSP)
tumours are pigmented.

Dermatofibrosarcoma Protuberans
• Rare- 1 case per million per year
• Cutaneous soft tissue sarcoma
originating from Fibroblasts
• 90% are low grade, 10% contain an
additional high grade sarcomatous
component therefore it is considered to
be of intermediate malignancy
• <4% of tumours can
metastasise/transform
fibrosarcomatous

Pathophysiology
• 90% of DFSP have the translocation between
Ch17 and Ch22 [t(17:22)]
• Creates a fusion between a collagen gene
and platelet derived growth

• Resultant fusion gene creates platelet
derived growth factor (PDGF)
• Fibroblasts carry the platelet-derived growth
factor receptor β
• Malignant cells produce PDGF which
stimulates fibroblast growth

• As malignant cells develop, tumour enlarges

Mortality/morbidity
• locally aggressive with high
recurrence rate
• 5-year survival - 99.2%
• metastasis rare (only 1-4%
reported)

Prognosis & Adjunct Therapy
• Tumour depth is the only factor
associated with disease- free survival
• Follow-up should involve
• Metastatic/ unresectable tumours can
be treated with Imatinib mesylate
(Glivec) if t(17:22) positive
• There is a role for radiotherapy after
surgery

Management
BB was referred to
the Skin Cancer MDT
for
further
management
On examination, he
had his original scar
on the right shoulder
as well as remnants
of his DFSP

Medical Treatment
• Radiation therapy
– margins positive
– Postoperative – reduce risk of recurrence

Excision
Wide Local Excision Vs.

Mohs Surgery

Goal is achieve clear surgical margins with some form of complete circumferential
and peripheral deep margin assessment before reconstruction

Management of DFSP

Matin, R.N., Acland, K.M. and Williams, H.C. (2012) British Journal of Dermatology,
167: 6–9.

No randomized controlled trials exist, probably due to the
rarity and varied presentation of DFSP.
A recent published review of the literature highlighted that
evidence for superiority of either procedure was limited.

Medical Treatment
• Imatinib
– inhibitor protein-tyrosine kinases
– platelet-derived growth factor (PDGF)
receptors
– unresectable, recurrent, metastatic DFSP
– preoperative therapy
• decrease tumour load
• promote tumour cell apoptosis
• reduce the extent of surgery.


Slide 10

Case 26: 25 y.o. Male. Right shoulder.
Keloid? Hypertrophic scar? Lichen simplex
chronicus?

On examination
On Examination
• Approximately 5x4cm
dermal nodule
• Firm texture
• right shoulder
• no lymphadenopathy.
The initial impression was this was a cyst and excision was organised by his
GP on a General Surgery Minor ops list.

Histopathology
• Unencapsulated spindle cell lesion
• composed of short fascicular arrays in a
storiform pattern
• nestled within the mid-dermis extending
widely into sub-cutis.
• Scattered melanophages were noted.
Immunolabelling showed strong positivity
for CD34 and very focal nonspecific
positivity for SMA & Factor XIIIa

CD34

S100

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma
Features consistent with a pigmented dermatofibrosarcoma
protuberans or Bednar tumour.

Bednar Tumour
Pigmented DFSP varies from ordinary DFSP by the presence of
non neoplastic melanin- laden dendritic cells; it is otherwise
morphologically and clinically identical to ordinary DFSP
Czech pathologist, Blahoslav
Bednar
(19161998),
first
described the Pigmented DFSP in
1957 while describing a group of
cutaneous tumours demonstrating
prominent storiform pattern and
melanin pigment which were
thought to be a variant of
Neurofibroma.

Less than 5% of dermatofibrosarcoma protuberans (DFSP)
tumours are pigmented.

Dermatofibrosarcoma Protuberans
• Rare- 1 case per million per year
• Cutaneous soft tissue sarcoma
originating from Fibroblasts
• 90% are low grade, 10% contain an
additional high grade sarcomatous
component therefore it is considered to
be of intermediate malignancy
• <4% of tumours can
metastasise/transform
fibrosarcomatous

Pathophysiology
• 90% of DFSP have the translocation between
Ch17 and Ch22 [t(17:22)]
• Creates a fusion between a collagen gene
and platelet derived growth

• Resultant fusion gene creates platelet
derived growth factor (PDGF)
• Fibroblasts carry the platelet-derived growth
factor receptor β
• Malignant cells produce PDGF which
stimulates fibroblast growth

• As malignant cells develop, tumour enlarges

Mortality/morbidity
• locally aggressive with high
recurrence rate
• 5-year survival - 99.2%
• metastasis rare (only 1-4%
reported)

Prognosis & Adjunct Therapy
• Tumour depth is the only factor
associated with disease- free survival
• Follow-up should involve
• Metastatic/ unresectable tumours can
be treated with Imatinib mesylate
(Glivec) if t(17:22) positive
• There is a role for radiotherapy after
surgery

Management
BB was referred to
the Skin Cancer MDT
for
further
management
On examination, he
had his original scar
on the right shoulder
as well as remnants
of his DFSP

Medical Treatment
• Radiation therapy
– margins positive
– Postoperative – reduce risk of recurrence

Excision
Wide Local Excision Vs.

Mohs Surgery

Goal is achieve clear surgical margins with some form of complete circumferential
and peripheral deep margin assessment before reconstruction

Management of DFSP

Matin, R.N., Acland, K.M. and Williams, H.C. (2012) British Journal of Dermatology,
167: 6–9.

No randomized controlled trials exist, probably due to the
rarity and varied presentation of DFSP.
A recent published review of the literature highlighted that
evidence for superiority of either procedure was limited.

Medical Treatment
• Imatinib
– inhibitor protein-tyrosine kinases
– platelet-derived growth factor (PDGF)
receptors
– unresectable, recurrent, metastatic DFSP
– preoperative therapy
• decrease tumour load
• promote tumour cell apoptosis
• reduce the extent of surgery.


Slide 11

Case 26: 25 y.o. Male. Right shoulder.
Keloid? Hypertrophic scar? Lichen simplex
chronicus?

On examination
On Examination
• Approximately 5x4cm
dermal nodule
• Firm texture
• right shoulder
• no lymphadenopathy.
The initial impression was this was a cyst and excision was organised by his
GP on a General Surgery Minor ops list.

Histopathology
• Unencapsulated spindle cell lesion
• composed of short fascicular arrays in a
storiform pattern
• nestled within the mid-dermis extending
widely into sub-cutis.
• Scattered melanophages were noted.
Immunolabelling showed strong positivity
for CD34 and very focal nonspecific
positivity for SMA & Factor XIIIa

CD34

S100

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma
Features consistent with a pigmented dermatofibrosarcoma
protuberans or Bednar tumour.

Bednar Tumour
Pigmented DFSP varies from ordinary DFSP by the presence of
non neoplastic melanin- laden dendritic cells; it is otherwise
morphologically and clinically identical to ordinary DFSP
Czech pathologist, Blahoslav
Bednar
(19161998),
first
described the Pigmented DFSP in
1957 while describing a group of
cutaneous tumours demonstrating
prominent storiform pattern and
melanin pigment which were
thought to be a variant of
Neurofibroma.

Less than 5% of dermatofibrosarcoma protuberans (DFSP)
tumours are pigmented.

Dermatofibrosarcoma Protuberans
• Rare- 1 case per million per year
• Cutaneous soft tissue sarcoma
originating from Fibroblasts
• 90% are low grade, 10% contain an
additional high grade sarcomatous
component therefore it is considered to
be of intermediate malignancy
• <4% of tumours can
metastasise/transform
fibrosarcomatous

Pathophysiology
• 90% of DFSP have the translocation between
Ch17 and Ch22 [t(17:22)]
• Creates a fusion between a collagen gene
and platelet derived growth

• Resultant fusion gene creates platelet
derived growth factor (PDGF)
• Fibroblasts carry the platelet-derived growth
factor receptor β
• Malignant cells produce PDGF which
stimulates fibroblast growth

• As malignant cells develop, tumour enlarges

Mortality/morbidity
• locally aggressive with high
recurrence rate
• 5-year survival - 99.2%
• metastasis rare (only 1-4%
reported)

Prognosis & Adjunct Therapy
• Tumour depth is the only factor
associated with disease- free survival
• Follow-up should involve
• Metastatic/ unresectable tumours can
be treated with Imatinib mesylate
(Glivec) if t(17:22) positive
• There is a role for radiotherapy after
surgery

Management
BB was referred to
the Skin Cancer MDT
for
further
management
On examination, he
had his original scar
on the right shoulder
as well as remnants
of his DFSP

Medical Treatment
• Radiation therapy
– margins positive
– Postoperative – reduce risk of recurrence

Excision
Wide Local Excision Vs.

Mohs Surgery

Goal is achieve clear surgical margins with some form of complete circumferential
and peripheral deep margin assessment before reconstruction

Management of DFSP

Matin, R.N., Acland, K.M. and Williams, H.C. (2012) British Journal of Dermatology,
167: 6–9.

No randomized controlled trials exist, probably due to the
rarity and varied presentation of DFSP.
A recent published review of the literature highlighted that
evidence for superiority of either procedure was limited.

Medical Treatment
• Imatinib
– inhibitor protein-tyrosine kinases
– platelet-derived growth factor (PDGF)
receptors
– unresectable, recurrent, metastatic DFSP
– preoperative therapy
• decrease tumour load
• promote tumour cell apoptosis
• reduce the extent of surgery.


Slide 12

Case 26: 25 y.o. Male. Right shoulder.
Keloid? Hypertrophic scar? Lichen simplex
chronicus?

On examination
On Examination
• Approximately 5x4cm
dermal nodule
• Firm texture
• right shoulder
• no lymphadenopathy.
The initial impression was this was a cyst and excision was organised by his
GP on a General Surgery Minor ops list.

Histopathology
• Unencapsulated spindle cell lesion
• composed of short fascicular arrays in a
storiform pattern
• nestled within the mid-dermis extending
widely into sub-cutis.
• Scattered melanophages were noted.
Immunolabelling showed strong positivity
for CD34 and very focal nonspecific
positivity for SMA & Factor XIIIa

CD34

S100

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma
Features consistent with a pigmented dermatofibrosarcoma
protuberans or Bednar tumour.

Bednar Tumour
Pigmented DFSP varies from ordinary DFSP by the presence of
non neoplastic melanin- laden dendritic cells; it is otherwise
morphologically and clinically identical to ordinary DFSP
Czech pathologist, Blahoslav
Bednar
(19161998),
first
described the Pigmented DFSP in
1957 while describing a group of
cutaneous tumours demonstrating
prominent storiform pattern and
melanin pigment which were
thought to be a variant of
Neurofibroma.

Less than 5% of dermatofibrosarcoma protuberans (DFSP)
tumours are pigmented.

Dermatofibrosarcoma Protuberans
• Rare- 1 case per million per year
• Cutaneous soft tissue sarcoma
originating from Fibroblasts
• 90% are low grade, 10% contain an
additional high grade sarcomatous
component therefore it is considered to
be of intermediate malignancy
• <4% of tumours can
metastasise/transform
fibrosarcomatous

Pathophysiology
• 90% of DFSP have the translocation between
Ch17 and Ch22 [t(17:22)]
• Creates a fusion between a collagen gene
and platelet derived growth

• Resultant fusion gene creates platelet
derived growth factor (PDGF)
• Fibroblasts carry the platelet-derived growth
factor receptor β
• Malignant cells produce PDGF which
stimulates fibroblast growth

• As malignant cells develop, tumour enlarges

Mortality/morbidity
• locally aggressive with high
recurrence rate
• 5-year survival - 99.2%
• metastasis rare (only 1-4%
reported)

Prognosis & Adjunct Therapy
• Tumour depth is the only factor
associated with disease- free survival
• Follow-up should involve
• Metastatic/ unresectable tumours can
be treated with Imatinib mesylate
(Glivec) if t(17:22) positive
• There is a role for radiotherapy after
surgery

Management
BB was referred to
the Skin Cancer MDT
for
further
management
On examination, he
had his original scar
on the right shoulder
as well as remnants
of his DFSP

Medical Treatment
• Radiation therapy
– margins positive
– Postoperative – reduce risk of recurrence

Excision
Wide Local Excision Vs.

Mohs Surgery

Goal is achieve clear surgical margins with some form of complete circumferential
and peripheral deep margin assessment before reconstruction

Management of DFSP

Matin, R.N., Acland, K.M. and Williams, H.C. (2012) British Journal of Dermatology,
167: 6–9.

No randomized controlled trials exist, probably due to the
rarity and varied presentation of DFSP.
A recent published review of the literature highlighted that
evidence for superiority of either procedure was limited.

Medical Treatment
• Imatinib
– inhibitor protein-tyrosine kinases
– platelet-derived growth factor (PDGF)
receptors
– unresectable, recurrent, metastatic DFSP
– preoperative therapy
• decrease tumour load
• promote tumour cell apoptosis
• reduce the extent of surgery.


Slide 13

Case 26: 25 y.o. Male. Right shoulder.
Keloid? Hypertrophic scar? Lichen simplex
chronicus?

On examination
On Examination
• Approximately 5x4cm
dermal nodule
• Firm texture
• right shoulder
• no lymphadenopathy.
The initial impression was this was a cyst and excision was organised by his
GP on a General Surgery Minor ops list.

Histopathology
• Unencapsulated spindle cell lesion
• composed of short fascicular arrays in a
storiform pattern
• nestled within the mid-dermis extending
widely into sub-cutis.
• Scattered melanophages were noted.
Immunolabelling showed strong positivity
for CD34 and very focal nonspecific
positivity for SMA & Factor XIIIa

CD34

S100

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma
Features consistent with a pigmented dermatofibrosarcoma
protuberans or Bednar tumour.

Bednar Tumour
Pigmented DFSP varies from ordinary DFSP by the presence of
non neoplastic melanin- laden dendritic cells; it is otherwise
morphologically and clinically identical to ordinary DFSP
Czech pathologist, Blahoslav
Bednar
(19161998),
first
described the Pigmented DFSP in
1957 while describing a group of
cutaneous tumours demonstrating
prominent storiform pattern and
melanin pigment which were
thought to be a variant of
Neurofibroma.

Less than 5% of dermatofibrosarcoma protuberans (DFSP)
tumours are pigmented.

Dermatofibrosarcoma Protuberans
• Rare- 1 case per million per year
• Cutaneous soft tissue sarcoma
originating from Fibroblasts
• 90% are low grade, 10% contain an
additional high grade sarcomatous
component therefore it is considered to
be of intermediate malignancy
• <4% of tumours can
metastasise/transform
fibrosarcomatous

Pathophysiology
• 90% of DFSP have the translocation between
Ch17 and Ch22 [t(17:22)]
• Creates a fusion between a collagen gene
and platelet derived growth

• Resultant fusion gene creates platelet
derived growth factor (PDGF)
• Fibroblasts carry the platelet-derived growth
factor receptor β
• Malignant cells produce PDGF which
stimulates fibroblast growth

• As malignant cells develop, tumour enlarges

Mortality/morbidity
• locally aggressive with high
recurrence rate
• 5-year survival - 99.2%
• metastasis rare (only 1-4%
reported)

Prognosis & Adjunct Therapy
• Tumour depth is the only factor
associated with disease- free survival
• Follow-up should involve
• Metastatic/ unresectable tumours can
be treated with Imatinib mesylate
(Glivec) if t(17:22) positive
• There is a role for radiotherapy after
surgery

Management
BB was referred to
the Skin Cancer MDT
for
further
management
On examination, he
had his original scar
on the right shoulder
as well as remnants
of his DFSP

Medical Treatment
• Radiation therapy
– margins positive
– Postoperative – reduce risk of recurrence

Excision
Wide Local Excision Vs.

Mohs Surgery

Goal is achieve clear surgical margins with some form of complete circumferential
and peripheral deep margin assessment before reconstruction

Management of DFSP

Matin, R.N., Acland, K.M. and Williams, H.C. (2012) British Journal of Dermatology,
167: 6–9.

No randomized controlled trials exist, probably due to the
rarity and varied presentation of DFSP.
A recent published review of the literature highlighted that
evidence for superiority of either procedure was limited.

Medical Treatment
• Imatinib
– inhibitor protein-tyrosine kinases
– platelet-derived growth factor (PDGF)
receptors
– unresectable, recurrent, metastatic DFSP
– preoperative therapy
• decrease tumour load
• promote tumour cell apoptosis
• reduce the extent of surgery.


Slide 14

Case 26: 25 y.o. Male. Right shoulder.
Keloid? Hypertrophic scar? Lichen simplex
chronicus?

On examination
On Examination
• Approximately 5x4cm
dermal nodule
• Firm texture
• right shoulder
• no lymphadenopathy.
The initial impression was this was a cyst and excision was organised by his
GP on a General Surgery Minor ops list.

Histopathology
• Unencapsulated spindle cell lesion
• composed of short fascicular arrays in a
storiform pattern
• nestled within the mid-dermis extending
widely into sub-cutis.
• Scattered melanophages were noted.
Immunolabelling showed strong positivity
for CD34 and very focal nonspecific
positivity for SMA & Factor XIIIa

CD34

S100

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma
Features consistent with a pigmented dermatofibrosarcoma
protuberans or Bednar tumour.

Bednar Tumour
Pigmented DFSP varies from ordinary DFSP by the presence of
non neoplastic melanin- laden dendritic cells; it is otherwise
morphologically and clinically identical to ordinary DFSP
Czech pathologist, Blahoslav
Bednar
(19161998),
first
described the Pigmented DFSP in
1957 while describing a group of
cutaneous tumours demonstrating
prominent storiform pattern and
melanin pigment which were
thought to be a variant of
Neurofibroma.

Less than 5% of dermatofibrosarcoma protuberans (DFSP)
tumours are pigmented.

Dermatofibrosarcoma Protuberans
• Rare- 1 case per million per year
• Cutaneous soft tissue sarcoma
originating from Fibroblasts
• 90% are low grade, 10% contain an
additional high grade sarcomatous
component therefore it is considered to
be of intermediate malignancy
• <4% of tumours can
metastasise/transform
fibrosarcomatous

Pathophysiology
• 90% of DFSP have the translocation between
Ch17 and Ch22 [t(17:22)]
• Creates a fusion between a collagen gene
and platelet derived growth

• Resultant fusion gene creates platelet
derived growth factor (PDGF)
• Fibroblasts carry the platelet-derived growth
factor receptor β
• Malignant cells produce PDGF which
stimulates fibroblast growth

• As malignant cells develop, tumour enlarges

Mortality/morbidity
• locally aggressive with high
recurrence rate
• 5-year survival - 99.2%
• metastasis rare (only 1-4%
reported)

Prognosis & Adjunct Therapy
• Tumour depth is the only factor
associated with disease- free survival
• Follow-up should involve
• Metastatic/ unresectable tumours can
be treated with Imatinib mesylate
(Glivec) if t(17:22) positive
• There is a role for radiotherapy after
surgery

Management
BB was referred to
the Skin Cancer MDT
for
further
management
On examination, he
had his original scar
on the right shoulder
as well as remnants
of his DFSP

Medical Treatment
• Radiation therapy
– margins positive
– Postoperative – reduce risk of recurrence

Excision
Wide Local Excision Vs.

Mohs Surgery

Goal is achieve clear surgical margins with some form of complete circumferential
and peripheral deep margin assessment before reconstruction

Management of DFSP

Matin, R.N., Acland, K.M. and Williams, H.C. (2012) British Journal of Dermatology,
167: 6–9.

No randomized controlled trials exist, probably due to the
rarity and varied presentation of DFSP.
A recent published review of the literature highlighted that
evidence for superiority of either procedure was limited.

Medical Treatment
• Imatinib
– inhibitor protein-tyrosine kinases
– platelet-derived growth factor (PDGF)
receptors
– unresectable, recurrent, metastatic DFSP
– preoperative therapy
• decrease tumour load
• promote tumour cell apoptosis
• reduce the extent of surgery.


Slide 15

Case 26: 25 y.o. Male. Right shoulder.
Keloid? Hypertrophic scar? Lichen simplex
chronicus?

On examination
On Examination
• Approximately 5x4cm
dermal nodule
• Firm texture
• right shoulder
• no lymphadenopathy.
The initial impression was this was a cyst and excision was organised by his
GP on a General Surgery Minor ops list.

Histopathology
• Unencapsulated spindle cell lesion
• composed of short fascicular arrays in a
storiform pattern
• nestled within the mid-dermis extending
widely into sub-cutis.
• Scattered melanophages were noted.
Immunolabelling showed strong positivity
for CD34 and very focal nonspecific
positivity for SMA & Factor XIIIa

CD34

S100

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma
Features consistent with a pigmented dermatofibrosarcoma
protuberans or Bednar tumour.

Bednar Tumour
Pigmented DFSP varies from ordinary DFSP by the presence of
non neoplastic melanin- laden dendritic cells; it is otherwise
morphologically and clinically identical to ordinary DFSP
Czech pathologist, Blahoslav
Bednar
(19161998),
first
described the Pigmented DFSP in
1957 while describing a group of
cutaneous tumours demonstrating
prominent storiform pattern and
melanin pigment which were
thought to be a variant of
Neurofibroma.

Less than 5% of dermatofibrosarcoma protuberans (DFSP)
tumours are pigmented.

Dermatofibrosarcoma Protuberans
• Rare- 1 case per million per year
• Cutaneous soft tissue sarcoma
originating from Fibroblasts
• 90% are low grade, 10% contain an
additional high grade sarcomatous
component therefore it is considered to
be of intermediate malignancy
• <4% of tumours can
metastasise/transform
fibrosarcomatous

Pathophysiology
• 90% of DFSP have the translocation between
Ch17 and Ch22 [t(17:22)]
• Creates a fusion between a collagen gene
and platelet derived growth

• Resultant fusion gene creates platelet
derived growth factor (PDGF)
• Fibroblasts carry the platelet-derived growth
factor receptor β
• Malignant cells produce PDGF which
stimulates fibroblast growth

• As malignant cells develop, tumour enlarges

Mortality/morbidity
• locally aggressive with high
recurrence rate
• 5-year survival - 99.2%
• metastasis rare (only 1-4%
reported)

Prognosis & Adjunct Therapy
• Tumour depth is the only factor
associated with disease- free survival
• Follow-up should involve
• Metastatic/ unresectable tumours can
be treated with Imatinib mesylate
(Glivec) if t(17:22) positive
• There is a role for radiotherapy after
surgery

Management
BB was referred to
the Skin Cancer MDT
for
further
management
On examination, he
had his original scar
on the right shoulder
as well as remnants
of his DFSP

Medical Treatment
• Radiation therapy
– margins positive
– Postoperative – reduce risk of recurrence

Excision
Wide Local Excision Vs.

Mohs Surgery

Goal is achieve clear surgical margins with some form of complete circumferential
and peripheral deep margin assessment before reconstruction

Management of DFSP

Matin, R.N., Acland, K.M. and Williams, H.C. (2012) British Journal of Dermatology,
167: 6–9.

No randomized controlled trials exist, probably due to the
rarity and varied presentation of DFSP.
A recent published review of the literature highlighted that
evidence for superiority of either procedure was limited.

Medical Treatment
• Imatinib
– inhibitor protein-tyrosine kinases
– platelet-derived growth factor (PDGF)
receptors
– unresectable, recurrent, metastatic DFSP
– preoperative therapy
• decrease tumour load
• promote tumour cell apoptosis
• reduce the extent of surgery.


Slide 16

Case 26: 25 y.o. Male. Right shoulder.
Keloid? Hypertrophic scar? Lichen simplex
chronicus?

On examination
On Examination
• Approximately 5x4cm
dermal nodule
• Firm texture
• right shoulder
• no lymphadenopathy.
The initial impression was this was a cyst and excision was organised by his
GP on a General Surgery Minor ops list.

Histopathology
• Unencapsulated spindle cell lesion
• composed of short fascicular arrays in a
storiform pattern
• nestled within the mid-dermis extending
widely into sub-cutis.
• Scattered melanophages were noted.
Immunolabelling showed strong positivity
for CD34 and very focal nonspecific
positivity for SMA & Factor XIIIa

CD34

S100

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma
Features consistent with a pigmented dermatofibrosarcoma
protuberans or Bednar tumour.

Bednar Tumour
Pigmented DFSP varies from ordinary DFSP by the presence of
non neoplastic melanin- laden dendritic cells; it is otherwise
morphologically and clinically identical to ordinary DFSP
Czech pathologist, Blahoslav
Bednar
(19161998),
first
described the Pigmented DFSP in
1957 while describing a group of
cutaneous tumours demonstrating
prominent storiform pattern and
melanin pigment which were
thought to be a variant of
Neurofibroma.

Less than 5% of dermatofibrosarcoma protuberans (DFSP)
tumours are pigmented.

Dermatofibrosarcoma Protuberans
• Rare- 1 case per million per year
• Cutaneous soft tissue sarcoma
originating from Fibroblasts
• 90% are low grade, 10% contain an
additional high grade sarcomatous
component therefore it is considered to
be of intermediate malignancy
• <4% of tumours can
metastasise/transform
fibrosarcomatous

Pathophysiology
• 90% of DFSP have the translocation between
Ch17 and Ch22 [t(17:22)]
• Creates a fusion between a collagen gene
and platelet derived growth

• Resultant fusion gene creates platelet
derived growth factor (PDGF)
• Fibroblasts carry the platelet-derived growth
factor receptor β
• Malignant cells produce PDGF which
stimulates fibroblast growth

• As malignant cells develop, tumour enlarges

Mortality/morbidity
• locally aggressive with high
recurrence rate
• 5-year survival - 99.2%
• metastasis rare (only 1-4%
reported)

Prognosis & Adjunct Therapy
• Tumour depth is the only factor
associated with disease- free survival
• Follow-up should involve
• Metastatic/ unresectable tumours can
be treated with Imatinib mesylate
(Glivec) if t(17:22) positive
• There is a role for radiotherapy after
surgery

Management
BB was referred to
the Skin Cancer MDT
for
further
management
On examination, he
had his original scar
on the right shoulder
as well as remnants
of his DFSP

Medical Treatment
• Radiation therapy
– margins positive
– Postoperative – reduce risk of recurrence

Excision
Wide Local Excision Vs.

Mohs Surgery

Goal is achieve clear surgical margins with some form of complete circumferential
and peripheral deep margin assessment before reconstruction

Management of DFSP

Matin, R.N., Acland, K.M. and Williams, H.C. (2012) British Journal of Dermatology,
167: 6–9.

No randomized controlled trials exist, probably due to the
rarity and varied presentation of DFSP.
A recent published review of the literature highlighted that
evidence for superiority of either procedure was limited.

Medical Treatment
• Imatinib
– inhibitor protein-tyrosine kinases
– platelet-derived growth factor (PDGF)
receptors
– unresectable, recurrent, metastatic DFSP
– preoperative therapy
• decrease tumour load
• promote tumour cell apoptosis
• reduce the extent of surgery.


Slide 17

Case 26: 25 y.o. Male. Right shoulder.
Keloid? Hypertrophic scar? Lichen simplex
chronicus?

On examination
On Examination
• Approximately 5x4cm
dermal nodule
• Firm texture
• right shoulder
• no lymphadenopathy.
The initial impression was this was a cyst and excision was organised by his
GP on a General Surgery Minor ops list.

Histopathology
• Unencapsulated spindle cell lesion
• composed of short fascicular arrays in a
storiform pattern
• nestled within the mid-dermis extending
widely into sub-cutis.
• Scattered melanophages were noted.
Immunolabelling showed strong positivity
for CD34 and very focal nonspecific
positivity for SMA & Factor XIIIa

CD34

S100

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma
Features consistent with a pigmented dermatofibrosarcoma
protuberans or Bednar tumour.

Bednar Tumour
Pigmented DFSP varies from ordinary DFSP by the presence of
non neoplastic melanin- laden dendritic cells; it is otherwise
morphologically and clinically identical to ordinary DFSP
Czech pathologist, Blahoslav
Bednar
(19161998),
first
described the Pigmented DFSP in
1957 while describing a group of
cutaneous tumours demonstrating
prominent storiform pattern and
melanin pigment which were
thought to be a variant of
Neurofibroma.

Less than 5% of dermatofibrosarcoma protuberans (DFSP)
tumours are pigmented.

Dermatofibrosarcoma Protuberans
• Rare- 1 case per million per year
• Cutaneous soft tissue sarcoma
originating from Fibroblasts
• 90% are low grade, 10% contain an
additional high grade sarcomatous
component therefore it is considered to
be of intermediate malignancy
• <4% of tumours can
metastasise/transform
fibrosarcomatous

Pathophysiology
• 90% of DFSP have the translocation between
Ch17 and Ch22 [t(17:22)]
• Creates a fusion between a collagen gene
and platelet derived growth

• Resultant fusion gene creates platelet
derived growth factor (PDGF)
• Fibroblasts carry the platelet-derived growth
factor receptor β
• Malignant cells produce PDGF which
stimulates fibroblast growth

• As malignant cells develop, tumour enlarges

Mortality/morbidity
• locally aggressive with high
recurrence rate
• 5-year survival - 99.2%
• metastasis rare (only 1-4%
reported)

Prognosis & Adjunct Therapy
• Tumour depth is the only factor
associated with disease- free survival
• Follow-up should involve
• Metastatic/ unresectable tumours can
be treated with Imatinib mesylate
(Glivec) if t(17:22) positive
• There is a role for radiotherapy after
surgery

Management
BB was referred to
the Skin Cancer MDT
for
further
management
On examination, he
had his original scar
on the right shoulder
as well as remnants
of his DFSP

Medical Treatment
• Radiation therapy
– margins positive
– Postoperative – reduce risk of recurrence

Excision
Wide Local Excision Vs.

Mohs Surgery

Goal is achieve clear surgical margins with some form of complete circumferential
and peripheral deep margin assessment before reconstruction

Management of DFSP

Matin, R.N., Acland, K.M. and Williams, H.C. (2012) British Journal of Dermatology,
167: 6–9.

No randomized controlled trials exist, probably due to the
rarity and varied presentation of DFSP.
A recent published review of the literature highlighted that
evidence for superiority of either procedure was limited.

Medical Treatment
• Imatinib
– inhibitor protein-tyrosine kinases
– platelet-derived growth factor (PDGF)
receptors
– unresectable, recurrent, metastatic DFSP
– preoperative therapy
• decrease tumour load
• promote tumour cell apoptosis
• reduce the extent of surgery.


Slide 18

Case 26: 25 y.o. Male. Right shoulder.
Keloid? Hypertrophic scar? Lichen simplex
chronicus?

On examination
On Examination
• Approximately 5x4cm
dermal nodule
• Firm texture
• right shoulder
• no lymphadenopathy.
The initial impression was this was a cyst and excision was organised by his
GP on a General Surgery Minor ops list.

Histopathology
• Unencapsulated spindle cell lesion
• composed of short fascicular arrays in a
storiform pattern
• nestled within the mid-dermis extending
widely into sub-cutis.
• Scattered melanophages were noted.
Immunolabelling showed strong positivity
for CD34 and very focal nonspecific
positivity for SMA & Factor XIIIa

CD34

S100

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma
Features consistent with a pigmented dermatofibrosarcoma
protuberans or Bednar tumour.

Bednar Tumour
Pigmented DFSP varies from ordinary DFSP by the presence of
non neoplastic melanin- laden dendritic cells; it is otherwise
morphologically and clinically identical to ordinary DFSP
Czech pathologist, Blahoslav
Bednar
(19161998),
first
described the Pigmented DFSP in
1957 while describing a group of
cutaneous tumours demonstrating
prominent storiform pattern and
melanin pigment which were
thought to be a variant of
Neurofibroma.

Less than 5% of dermatofibrosarcoma protuberans (DFSP)
tumours are pigmented.

Dermatofibrosarcoma Protuberans
• Rare- 1 case per million per year
• Cutaneous soft tissue sarcoma
originating from Fibroblasts
• 90% are low grade, 10% contain an
additional high grade sarcomatous
component therefore it is considered to
be of intermediate malignancy
• <4% of tumours can
metastasise/transform
fibrosarcomatous

Pathophysiology
• 90% of DFSP have the translocation between
Ch17 and Ch22 [t(17:22)]
• Creates a fusion between a collagen gene
and platelet derived growth

• Resultant fusion gene creates platelet
derived growth factor (PDGF)
• Fibroblasts carry the platelet-derived growth
factor receptor β
• Malignant cells produce PDGF which
stimulates fibroblast growth

• As malignant cells develop, tumour enlarges

Mortality/morbidity
• locally aggressive with high
recurrence rate
• 5-year survival - 99.2%
• metastasis rare (only 1-4%
reported)

Prognosis & Adjunct Therapy
• Tumour depth is the only factor
associated with disease- free survival
• Follow-up should involve
• Metastatic/ unresectable tumours can
be treated with Imatinib mesylate
(Glivec) if t(17:22) positive
• There is a role for radiotherapy after
surgery

Management
BB was referred to
the Skin Cancer MDT
for
further
management
On examination, he
had his original scar
on the right shoulder
as well as remnants
of his DFSP

Medical Treatment
• Radiation therapy
– margins positive
– Postoperative – reduce risk of recurrence

Excision
Wide Local Excision Vs.

Mohs Surgery

Goal is achieve clear surgical margins with some form of complete circumferential
and peripheral deep margin assessment before reconstruction

Management of DFSP

Matin, R.N., Acland, K.M. and Williams, H.C. (2012) British Journal of Dermatology,
167: 6–9.

No randomized controlled trials exist, probably due to the
rarity and varied presentation of DFSP.
A recent published review of the literature highlighted that
evidence for superiority of either procedure was limited.

Medical Treatment
• Imatinib
– inhibitor protein-tyrosine kinases
– platelet-derived growth factor (PDGF)
receptors
– unresectable, recurrent, metastatic DFSP
– preoperative therapy
• decrease tumour load
• promote tumour cell apoptosis
• reduce the extent of surgery.


Slide 19

Case 26: 25 y.o. Male. Right shoulder.
Keloid? Hypertrophic scar? Lichen simplex
chronicus?

On examination
On Examination
• Approximately 5x4cm
dermal nodule
• Firm texture
• right shoulder
• no lymphadenopathy.
The initial impression was this was a cyst and excision was organised by his
GP on a General Surgery Minor ops list.

Histopathology
• Unencapsulated spindle cell lesion
• composed of short fascicular arrays in a
storiform pattern
• nestled within the mid-dermis extending
widely into sub-cutis.
• Scattered melanophages were noted.
Immunolabelling showed strong positivity
for CD34 and very focal nonspecific
positivity for SMA & Factor XIIIa

CD34

S100

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma
Features consistent with a pigmented dermatofibrosarcoma
protuberans or Bednar tumour.

Bednar Tumour
Pigmented DFSP varies from ordinary DFSP by the presence of
non neoplastic melanin- laden dendritic cells; it is otherwise
morphologically and clinically identical to ordinary DFSP
Czech pathologist, Blahoslav
Bednar
(19161998),
first
described the Pigmented DFSP in
1957 while describing a group of
cutaneous tumours demonstrating
prominent storiform pattern and
melanin pigment which were
thought to be a variant of
Neurofibroma.

Less than 5% of dermatofibrosarcoma protuberans (DFSP)
tumours are pigmented.

Dermatofibrosarcoma Protuberans
• Rare- 1 case per million per year
• Cutaneous soft tissue sarcoma
originating from Fibroblasts
• 90% are low grade, 10% contain an
additional high grade sarcomatous
component therefore it is considered to
be of intermediate malignancy
• <4% of tumours can
metastasise/transform
fibrosarcomatous

Pathophysiology
• 90% of DFSP have the translocation between
Ch17 and Ch22 [t(17:22)]
• Creates a fusion between a collagen gene
and platelet derived growth

• Resultant fusion gene creates platelet
derived growth factor (PDGF)
• Fibroblasts carry the platelet-derived growth
factor receptor β
• Malignant cells produce PDGF which
stimulates fibroblast growth

• As malignant cells develop, tumour enlarges

Mortality/morbidity
• locally aggressive with high
recurrence rate
• 5-year survival - 99.2%
• metastasis rare (only 1-4%
reported)

Prognosis & Adjunct Therapy
• Tumour depth is the only factor
associated with disease- free survival
• Follow-up should involve
• Metastatic/ unresectable tumours can
be treated with Imatinib mesylate
(Glivec) if t(17:22) positive
• There is a role for radiotherapy after
surgery

Management
BB was referred to
the Skin Cancer MDT
for
further
management
On examination, he
had his original scar
on the right shoulder
as well as remnants
of his DFSP

Medical Treatment
• Radiation therapy
– margins positive
– Postoperative – reduce risk of recurrence

Excision
Wide Local Excision Vs.

Mohs Surgery

Goal is achieve clear surgical margins with some form of complete circumferential
and peripheral deep margin assessment before reconstruction

Management of DFSP

Matin, R.N., Acland, K.M. and Williams, H.C. (2012) British Journal of Dermatology,
167: 6–9.

No randomized controlled trials exist, probably due to the
rarity and varied presentation of DFSP.
A recent published review of the literature highlighted that
evidence for superiority of either procedure was limited.

Medical Treatment
• Imatinib
– inhibitor protein-tyrosine kinases
– platelet-derived growth factor (PDGF)
receptors
– unresectable, recurrent, metastatic DFSP
– preoperative therapy
• decrease tumour load
• promote tumour cell apoptosis
• reduce the extent of surgery.


Slide 20

Case 26: 25 y.o. Male. Right shoulder.
Keloid? Hypertrophic scar? Lichen simplex
chronicus?

On examination
On Examination
• Approximately 5x4cm
dermal nodule
• Firm texture
• right shoulder
• no lymphadenopathy.
The initial impression was this was a cyst and excision was organised by his
GP on a General Surgery Minor ops list.

Histopathology
• Unencapsulated spindle cell lesion
• composed of short fascicular arrays in a
storiform pattern
• nestled within the mid-dermis extending
widely into sub-cutis.
• Scattered melanophages were noted.
Immunolabelling showed strong positivity
for CD34 and very focal nonspecific
positivity for SMA & Factor XIIIa

CD34

S100

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma

CASE 26

• A. Hypertrophic scar
• B. Fibroma
• C. Perineuroma
• D. Dermatofibrosarcoma
• E. Dermatomyofibroma
Features consistent with a pigmented dermatofibrosarcoma
protuberans or Bednar tumour.

Bednar Tumour
Pigmented DFSP varies from ordinary DFSP by the presence of
non neoplastic melanin- laden dendritic cells; it is otherwise
morphologically and clinically identical to ordinary DFSP
Czech pathologist, Blahoslav
Bednar
(19161998),
first
described the Pigmented DFSP in
1957 while describing a group of
cutaneous tumours demonstrating
prominent storiform pattern and
melanin pigment which were
thought to be a variant of
Neurofibroma.

Less than 5% of dermatofibrosarcoma protuberans (DFSP)
tumours are pigmented.

Dermatofibrosarcoma Protuberans
• Rare- 1 case per million per year
• Cutaneous soft tissue sarcoma
originating from Fibroblasts
• 90% are low grade, 10% contain an
additional high grade sarcomatous
component therefore it is considered to
be of intermediate malignancy
• <4% of tumours can
metastasise/transform
fibrosarcomatous

Pathophysiology
• 90% of DFSP have the translocation between
Ch17 and Ch22 [t(17:22)]
• Creates a fusion between a collagen gene
and platelet derived growth

• Resultant fusion gene creates platelet
derived growth factor (PDGF)
• Fibroblasts carry the platelet-derived growth
factor receptor β
• Malignant cells produce PDGF which
stimulates fibroblast growth

• As malignant cells develop, tumour enlarges

Mortality/morbidity
• locally aggressive with high
recurrence rate
• 5-year survival - 99.2%
• metastasis rare (only 1-4%
reported)

Prognosis & Adjunct Therapy
• Tumour depth is the only factor
associated with disease- free survival
• Follow-up should involve
• Metastatic/ unresectable tumours can
be treated with Imatinib mesylate
(Glivec) if t(17:22) positive
• There is a role for radiotherapy after
surgery

Management
BB was referred to
the Skin Cancer MDT
for
further
management
On examination, he
had his original scar
on the right shoulder
as well as remnants
of his DFSP

Medical Treatment
• Radiation therapy
– margins positive
– Postoperative – reduce risk of recurrence

Excision
Wide Local Excision Vs.

Mohs Surgery

Goal is achieve clear surgical margins with some form of complete circumferential
and peripheral deep margin assessment before reconstruction

Management of DFSP

Matin, R.N., Acland, K.M. and Williams, H.C. (2012) British Journal of Dermatology,
167: 6–9.

No randomized controlled trials exist, probably due to the
rarity and varied presentation of DFSP.
A recent published review of the literature highlighted that
evidence for superiority of either procedure was limited.

Medical Treatment
• Imatinib
– inhibitor protein-tyrosine kinases
– platelet-derived growth factor (PDGF)
receptors
– unresectable, recurrent, metastatic DFSP
– preoperative therapy
• decrease tumour load
• promote tumour cell apoptosis
• reduce the extent of surgery.